Extra-medullary Hematopoiesis Causing Bilateral Optic Atrophy in Beta Thalassemia/Hb E Disease
Nimitr Ittipunkul MD*, Tada Martin MD*, Rutchada Siriwanasan MD*, Kanokporn Olanratanachai MD**, Jack Rootman MD***
Affiliation : * Department of Ophthalmology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand ** Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand *** Department of Ophthalmology, Faculty of Medicine, University of British Columbia, Vancouver, Canada
Objective : To report a rare presentation of bilateral optic nerve compression from extra-medullary hemato-
poiesis in beta thalassemia/Hb E disease.
Design : Interventional case report.
Clinical presentation: A 13-year-old Thai girl was reported with slowly progressive bilateral visual loss due
to optic neuropathy as a result of compression from extra-medullary hematopoiesis. Computed tomography of
orbit and brain revealed a homogeneous enhancing midline soft tissue mass originating from the ethmoid and
sphenoid sinuses along with extensive involvement of the skull.
Intervention: Blood transfusion once every month and low-dose radiotherapy to sphenoid and ethmoid bones
with some visual improvement.
Conclusion : Extra-medullary hematopoiesis in beta thalassemia can involve paranasal sinuses and cause
compressive neuropathy. In patients with beta thalassemia that required multiple blood transfusions with
progressive visual loss, ectopic marrow compression of optic nerve should be ruled out.
Keywords : Optic atrophy, Blurred vision, Extramedullary hematopoiesis, Thalassemia
